Our results conflict with those from a recent study describing no increased rate of rupture during pregnancy. This difference may reflect unique population attributes influencing brain AVM hemorrhage during pregnancy.

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OBJECTIVESickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors’ aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy.METHODSThe authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013. Pediatric patients (age < 18 years) with confirmed diagnoses of SCD and MMS were included. Intracranial stroke occurrence during the follow-up period was compared between surgically and conservatively managed patients.RESULTSA total of 15 pediatric SCD-MMS patients (28 affected hemispheres) were included in this study, and all were African American. Seven patients (12 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 ± 4.0 years, and 9 patients (60.0%) were female. Fourteen patients (93.3%) had strokes before diagnosis of MMS, with an average age at first stroke of 6.6 ± 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres, whereas no patient undergoing the revascularization procedure had any strokes at follow-up (p = 0.029). Three patients experienced immediate postoperative transient ischemic attacks, but all recovered without subsequent strokes.CONCLUSIONSIndirect revascularization is suggested as a safe and effective alternative to the best medical therapy alone in patients with SCD-MMS. High-risk patients managed on a regimen of chronic transfusion should be considered for indirect revascularization to maximize the effect of stroke prevention.

Background. Primary intramedullary spinal cord lymphoma (PISCL) is a rare diagnosis with poorly understood disease progression. Clarification of the factors associated with survival in PISCL patients is warranted. Methods. We conducted a population-based cohort study utilizing prospectively collected data from the Surveillance, Epidemiology, and End Results (SEER) database. Patients with histological diagnosis of primary lymphoma in spinal cord (C72.0) from 1973 to 2012 in the SEER database were included. Multivariable survival analysis between patient, lesion characteristics, and PISCL-related death was performed to adjust for confounding factors. Results. We included 346 PISCL patients in our study. Average age was 56.5 ± 17.8 years, with 62.7% being male. Racial distribution of these patients was white (87.6%), black (8.0%), and other (4.3%). More than half (55.8%) of patients were married. The most prevalent histology of PISCL was diffuse B-cell (46.2%), and the majority (55.2%) were low stage (Ann Arbor stage I/II). Most patients (67.9%) received radiation therapy. Average survival interval of patients with PISCL-related death (n=135, 39.0%) was 27.8 months. General cumulative survival probability at 1 year, 2 years, and 5 years was 73.8%, 67.9%, and 63.1%, respectively. Multivariable accelerated failure time (AFT) regression showed follicular lymphoma (HR:0.25, P=.008) and more recent diagnosis (HR:0.96, P<.001) was positively associated with PISCL-related survival. Conversely, nonwhite race (HR:1.69, P=.046), older age (HR:1.02, P<.001), unmarried status (HR:2.14, P<.001), and higher stage (HR:1.54, P=.022) were negatively associated with survival. Conclusions. Age, race, marital status, tumor histology, tumor stage, and year of diagnosis were associated with survival of PISCL. While most PISCL-related deaths occur within a 1-year period, subsequent slow progression was observed after the first year of survival.

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OBJECTIVE Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms whose treatment is often technically challenging. Given the low volume seen at most centers, perioperative outcomes have been reported infrequently. Here, the authors present the largest single-institution series of IMSCTs, focusing on the clinical presentation, histological makeup, perioperative outcomes, and long-term survival of surgically treated patients. METHODS A cohort of patients operated on for primary IMSCTs at a comprehensive cancer center between June 2002 and May 2020 was retrospectively identified. Data on patient demographics, tumor histology, neuraxial location, baseline neurological status, functional deficits, and operative characteristics were collected. Perioperative outcomes of interest included length of stay, postoperative complications, readmission, reoperation, and discharge disposition. Data were compared across tumor histologies using the Kruskal-Wallis H test, chi-square test, and Fisher exact test. Pairwise comparisons were conducted using Tukey’s honest significant difference test, chi-square test, and Fisher exact test. Long-term survival was assessed across tumor categories and histological subtype using the log-rank test. RESULTS Three hundred two patients were included in the study (mean age 34.9 ± 19 years, 77% white, 57% male). The most common tumors were ependymomas (47%), astrocytomas (31%), and hemangioblastomas (11%). Ependymomas and hemangioblastomas disproportionately localized to the cervical cord (54% and 59%, respectively), whereas astrocytomas were distributed almost equally between the cervical cord (36%) and thoracic cord (38%). Clinical presentation, extent of functional dependence, and postoperative 30-day outcomes were largely independent of underlying tumor pathology, although tumors of the thoracic cord had worse American Spinal Injury Association (ASIA) grades than cervical tumors. Rates of gross-total resection were lower for astrocytomas than for ependymomas (54% vs 84%, p < 0.01) and hemangioblastomas (54% vs 100%, p < 0.01). Additionally, 30-day readmission rates were significantly higher for astrocytomas than ependymomas (14% vs 6%, p = 0.02). Overall survival was significantly affected by the underlying pathology, with astrocytomas having poorer associated prognoses (40% at 15 years) than ependymomas (81%) and hemangioblastomas (66%; p < 0.01) and patients with high-grade ependymomas and astrocytomas having poorer long-term survival than those with low-grade lesions (p < 0.01). CONCLUSIONS The neuraxial location of IMSCTs, extent of resection, and postoperative survival differed significantly across tumor pathologies. However, perioperative outcomes did not vary significantly across tumor cohorts, suggesting that operative details, rather than pathology, may have a stronger influence on the short-term clinical course, whereas pathology appears to have a stronger impact on long-term survival.

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